Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease

The spectrum of imaging manifestations of Gorham-Stout disease: a novel dynamic contrast-enhanced MR lymphangiography

Yuna Lee, Seunghyun Lee, Saebeom Hur, Yun Soo Jeong, Dong In Suh, Jangsup Moon, Man Jin Kim, Young Hun Choi, Jung‑Eun CheonSeoul National University Hospital. Seoul National University College of Medicine. Republic of Korea Orphanet Journal of Rare DiseasesOrphanet J Rare Dis 2023; 18: DOI: 10.1186/s13023-023-02704-7 AbstractBackground: To describe the radiological features of Gorham-Stout disease (GSD) as […]

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Brain Abscess and Stroke in Children and Adults With Hereditary Hemorrhagic Telangiectasia: Analysis of a Large National Claims Database

Andrew J. White, Itay Marmor, Kate M. Peacock, Katelin B. Nickel, Jessica Zavadil, Margaret A. OlsenSaint Louis University School of Medicine. Washington University School of Medicine.United States NeurologyNeurology 2023; DOI: 10.1212/WNL.0000000000207269 AbstractObjective: Hereditary hemorrhagic telangiectasia (HHT) is an inherited disease associated with pathogenic variants in TGF-β signaling pathway-related genes, resulting in abnormal vascular development in various organs.

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Systemic artery to pulmonary artery aneurysm malformations associated with variants at MCF2L

S.E. Mitchell, R. P. Martin, P. Terry, S. E. Drant, D. Valle, H. Dietz, N. SobreiraJohns Hopkins University School of Medicine. Children’s Hospital of Philadelphia. United States American Journal of Medical GeneticsAm J Med Genet 2023; 191: 1250-1260DOI: 10.1002/ajmg.a.63141 AbstractArteriovenous malformations (AVM) are characterized by abnormal vessels connecting arteries and veins resulting in a disruption of

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First Genotype-Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease

Matina Prapa, Mauro Lago-Docampo, Emilia M. Swietlik, David Montani, Melanie Eyries, Marc Humbert, Carrie L. Welch, Wendy K. Chung, Rolf M. F. Berger, Harm Jan Bogaard, Olivier Danhaive, Pilar Escribano-Subıas, Henning Gall, Barbara Girerd, Ignacio Hernandez-Gonzalez, Simon Holden, David Hunt, Samara M. A. Jansen, Wilhelmina Kerstjens-Frederikse, David G. Kiely, Pablo Lapunzina, John McDermott, Shahin Moledina,

First Genotype-Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease Read More »

Physiologically based pharmacokinetic modelling of treprostinil after intravenous injection and extended-release oral tablet administration in healthy volunteers: An extrapolation to other patient populations including patients with hepatic impairment

Xuemei Wu, Xiaohan Zhang, Ruichao Xu, Imam Hussain Shaik, Raman VenkataramananUniversity of Pittsburgh School of Pharmacy. Fujian Medical University Union Hospital. University of Virginia. University of Pittsburgh School of Medicine.United States and China British Journal of Clinical PharmacologyBrit J Clin Pharmacol 2022; 88: 587-599DOI: 10.1111/bcp.14966 AbstractAims: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary arterial pressure,

Physiologically based pharmacokinetic modelling of treprostinil after intravenous injection and extended-release oral tablet administration in healthy volunteers: An extrapolation to other patient populations including patients with hepatic impairment Read More »

Construction and Evaluation of a Bio-Engineered Pump to Enable Subpulmonary Support of the Fontan Circulation: A Proof-of-Concept Study

Christopher R. Broda, P. Alex Smith, Yaxin Wang, Hamsini Sriraman, Peter S. Owuor, Luiz C. Sampaio, Iki Adachi, Doris A. Taylor, Christopher R. Broda, P. Alex Smith, Yaxin Wang, Hamsini Sriraman, Peter S. OwuorBaylor College of Medicine and Texas Children’s Hospital. Texas Medical Center. Texas Heart Institute. Pennsylvania State University. University of Texas Health Science

Construction and Evaluation of a Bio-Engineered Pump to Enable Subpulmonary Support of the Fontan Circulation: A Proof-of-Concept Study Read More »

Cardiovascular magnetic resonance in autoimmune rheumatic diseases: a clinical consensus document by the European Association of Cardiovascular Imaging

S. Mavrogeni, A. Pepe, K. R. Nijveldt, N. Ntusi, L.M. Sierra-Galan, Bratis, J. Wei, M. Mukherjee, G. Markousis-Mavrogenis, L. Gargani, L.E. Sade, N. Ajmone-Marsan, M. P. Seferovic, E. Donal, Nurmohamed, M. Matucci Cerinic, P. Sfikakis, G. Kitas, J. Schwitter, J. A. C. Lima, Dana Dawson, Marc Dweck, Kristina H. Haugaa, Niall Keenan, James Moon, Ivan

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Cardiac Fibrosis in the Pressure Overloaded Left and Right Ventricle as a Therapeutic Target

Katharina Schimmel, Kenzo Ichimura, Sushma Reddy, Francois Haddad, Edda SpiekerkoetterStanford University School of Medicine. United States Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2022; 9: DOI: 10.3389/fcvm.2022.886553 AbstractMyocardial fibrosis is a remodeling process of the extracellular matrix (ECM) following cardiac stress. “Replacement fibrosis” is a term used to describe wound healing in the acute phase of an

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Effects of a Long-Term Wearable Activity Tracker-Based Exercise Intervention on Cardiac Morphology and Function of Patients with Cystic Fibrosis

Maria Anifanti, Stavros Giannakoulakos, Elpis Hatziagorou, Asterios Kampouras, John Tsanakas, Asterios Deligiannis, Evangelia KouidiAristotle University of Thessaloniki and Hippokration Hospital. Greece SensorsSensors 2022; 22: DOI: 10.3390/s22134884 AbstractSeveral studies have shown that patients with cystic fibrosis (CF), even at a young age, have pulmonary and cardiac abnormalities. The main complications are cardiac right ventricular (RV) systolic and/or

Effects of a Long-Term Wearable Activity Tracker-Based Exercise Intervention on Cardiac Morphology and Function of Patients with Cystic Fibrosis Read More »

Natural history of Myhre syndrome

David Dawei Yang, Marlene Rio, Caroline Michot, Nathalie Boddaert, Wael Yacoub, Nicolas Garcelon, Briac Thierry, Damien Bonnet, Sophie Rondeau, Dominique Herve, Stephanie Guey, Francois Angoulvant, Valerie Cormier‑DaireUniversité de Paris. Hôpital Universitaire Necker-Enfants Malades. INSERM U. Hôpital Lariboisière. France Orphanet Journal of Rare DiseasesOrphanet J Rare Dis 2022; 17: DOI: 10.1186/s13023-022-02447-x AbstractBackground: Myhre syndrome (MS) is a rare

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